Fact-sheet: Pheochromocytoma
Updated on 10/05/2016 at 2:00 PM
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Definition
Adrenal pheochromocytoma: rare chromaffin cell adrenal tumor secreting catecholamines.
Clinical features
Clinical features:
paroxysmal or sustained hypertension
Ménard's triad: headaches, sweating, and palpitations
Laboratory findings
Elevated 24-hour urinary metanephrines and normetanephrines
Elevated chromogranin A
CT
Sensitivity 93-100%
Solid adrenal mass, which may be cystic or heterogeneous
Marked enhancement, but contrast injection may trigger a hypertensive crisis (use non-ionic contrast medium)
MRI
T1: iso- or mildly hypointense relative to the liver
Possible hemorrhagic areas: spontaneous and heterogeneous T1 hyperintensity with T2 hypointensity
Marked T2 hyperintensity of cystic areas
Intense enhancement following gadolinium injection
No signal change on IP/OP sequences
Nuclear medicine
I-131/I-123 MIBG scintigraphy
Indicated when CT/MRI are normal to detect an extra-adrenal pheochromocytoma
Management
Curative surgical treatment with histologic confirmation
Biopsy is contraindicated due to the risk of catecholamine release